Objective To explore the status quo of nursing practice of hemophilia-specialized nurses, to find out the shortcomings of current work and to provide the basis for promoting the development of hemophilia-specialized nursing in China. Methods The nursing staff attending the National Nursing Conference on Hemophilia in Shenzhen on November 7, 2015 were investigated with questionnaires. This investigation involved more than 40 hemophilia treatment centers nationwide. The nursing staff were required to fill out the Questionnaire for Hemophilia-Specialized Nurses developed by the Nursing Council, World Hemophilia Federation on the site, which were taken back once completed. Totally 54 questionnaires were handed out, and 43 valid questionnaires were taken back. Results Totally 55.8% of the nurses engaged in hemophilia nursing for less than 5 years, and 58.1% of them spent less than 10 hours on hemophilia nursing per week. And the number of hemophilia centers whose requirement for specialized nurses was a college degree accounted for 55.8%. The major nursing practice of China's hemophilia-specialized nurses included preliminary diagnosis-related content and self-injection training (>50%), and involved less specialized nursing practice, including consultancy, education, liaison, triage, physical examination, medication management, extended care, nursing research and clinical trial (20%-50%). The functions of management and medical advice accounted for<20%, and the least nursing practice was "school visit", accounting for only 4.7%. Merely 5 hemophilia centers carried out nursing practice items that accounted for over 50%. Nurses with different working years and education backgrounds were moderately correlated with the number of carried out nursing practice items. Conclusions The hemophilia-specialized nurses in China has an uneven level, and they need to expand the nursing work, do better in information registration and health education for hemophilia patients, improve the training proportion of family treatment, and meanwhile strengthen the publicity of hemophilia in elementary and middle schools.

Objective To explore the joint health of children with haemophilia and its influencing factors.Methods Sixty boys with haemophilia (aged 4-18 years) were recruited from Peking Union Medical College Hospital.They were divided by age into a five to seven year-old group (group A,n=16),an eight to twelve year-old group (group B,n =18) and a thirteen to eighteen year-old group (group C,n =26).The haemophilia joint health score (HJHS) (version 2.1) was used to evaluate the condition of their elbows,knees and ankles.Spearman's method was applied to analyze the influencing factors.Results The average HJHSs of the knees,elbows and ankles of all of subjects were 8.65±6.41,5.30±5.30,and 5.40±4.25 respectively.The average for the knees was significantly higher than for the elbows and ankles.The average HJHS for the elbows of group A was significantly lower than that of their knees.The average HJHS for their knees was not significantly different from that of their ankles.For those aged 13-18 years,the average HJHS for their knees was significantly higher than that for their elbows and ankles.There was no significant difference between the HJHSs for the elbows,knees and ankles of the 8-12 year-olds.The HJHSs were positively correlated with age but negatively correlated with average therapeutic dose and exercise.Conclusion The joint health of haemophilic boys,especially that of the knees,decreases with age.Their joint health can be improved by medication and by taking reasonable physical exercise.

  Objective  To investigate the incidence and adverse effects of abnormal uterine bleeding (AUB) during oral anticoagulation therapy in women with thrombophilia.  Methods  The female patients with thrombophilia who received oral anticoagulation therapy with rivaroxaban or warfarin in the Department of Hematology of Peking Union Medical College Hospital from January 2013 to May 2023 were selected as the study subjects. Their demographic characteristics, disease related data and AUB before and after anticoagulant therapy were retrospectively collected. The patients were divided into rivaroxaban treatment group and warfarin treatment group according to anticoagulant drugs. The generalized estimating equation was used to analyze the difference in the incidence of AUB before and after anticoagulant therapy, and explore the effect of different anticoagulant therapy on AUB.  Results  A total of 106 female patients with thrombophilia were included, and we found that oral anticoagulation significantly increased the incidence of AUB (56.6% vs. 26.4%, P < 0.001), predominantly characterized by excessive menstruation (48.1%) and prolonged periods (21.7%). Rivaroxaban was more likely to cause AUB than warfarin (OR=3.3, 95% CI: 1.5-7.4, P=0.003). Of the patients who experienced excessive menstruation and prolonged periods, 72.2%(39/54) required intervention, with suspension of anticoagulants during menstruation as the main intervention (37.0%). The patients taking rivaroxaban were more likely to stop taking them during menstruation compared to those taking warfarin (OR=10.4, 95% CI: 1.2-87.2, P=0.019).  Conclusions  Oral anticoagulation therapy significantly increased the incidence of AUB in women with thrombophilia and the risk of AUB associated with rivaroxaban was significantly higher than that with warfarin.

A young male diagnosed with severe hemophilia A since childhood, was presented with recurrent joint and urinary bleeding. Annualized bleed rates dropped below five with low dose prophylactic medication.Bleeding in the right knee joint recently aggravated. Due to coexisting HIV infection and advanced hemophilic arthritis, the patient was managed by a multi-disciplinary team(MDT).Total knee arthroplasty was performed by an experienced surgeon using modern prosthesis design and intraoperative navigation technologies.Physical and rehabilitation therapy was provided during the postoperative period, and joint function improved. The MDT managed the young patient with HIV infection and advanced hemophilic arthritis. The patient was diagnosed with osteoporosis thought to have been caused by hemophilia, HIV infection and antiviral drugs; and he received treatment. The treatment of this patient reflects the importance of multidisciplinary cooperation in the management of difficult and rare diseases.